Challenging Management of Granulomatosis with Polyangiitis in a Patient with Multiorgan Involvement and Persistent Disease Activity Despite CD-19 Depletion

Author(s): FNU Warsha, Supriya peshin, Fnu Vineesha, Dilpat Kumar, Fnu Sindhu

Granulomatosis with polyangiitis (GPA) is a complex ANCA-associated vasculitis that presents significant challenges in management, particularly in patients with extensive multi-organ involvement and comorbidities. This case details a 41-year-old female with a long-standing history of GPA diagnosed at age 13, exhibiting severe manifestations including respiratory, renal, and ocular symptoms, alongside untreated atrial fibrillation and coronary artery disease. Following a hiatus from immunosuppressive therapy, the patient experienced exacerbation of her condition, characterized by cavitary lung lesions and recurrent infections. Diagnostic challenges were compounded by variable symptomatology, including neurological deficits and skin manifestations. Treatment strategies included high-dose corticosteroids, intravenous antibiotics, and the resumption of rituximab and cyclophosphamide therapy. Despite a tailored approach, the patient's condition highlighted the unpredictability of treatment responses and the need for continuous monitoring. This case underscores the necessity for a multidisciplinary approach in managing GPA, focusing on individualized treatment plans that address both the disease and its complications. Future research should aim to identify biomarkers for disease activity to enhance prognostic accuracy and refine management strategies for patients with GPA and similar autoimmune disorders.